Luigetti Marco, Guglielmino Valeria, Romozzi Marina, Romano Angela, Di Paolantonio Andrea, Bisogni Giulia, Sabatelli Eleonora, Modoni Anna, Sabatelli Mario, Servidei Serenella, Lo Monaco Mauro
Fondazione Policlinico Universitario A. Gemelli IRCCS, UOC Neurologia, 00168 Rome, Italy.
Dipartimento Di Neuroscienze, Università Cattolica del Sacro Cuore, 00168 Rome, Italy.
Brain Sci. 2022 Aug 4;12(8):1037. doi: 10.3390/brainsci12081037.
The objective of the study is to provide age-related normative values for dorsal sural nerve (DSN) and to analyse its application during follow-up of hereditary transthyretin amyloidosis (ATTRv) pre-symptomatic subjects. We consecutively recruited ATTRv pre-symptomatic carriers in which clinical examination, cardiological evaluation, and nerve conduction studies of the sural nerve and DSN were performed. To provide normative data of DSN, neurophysiologic parameters from healthy controls referred to our service were entered into linear regression analyses to check the relative influence of age and height. A correction grid was then derived. We collected 231 healthy subjects: the mean DSN sensory nerve action potential (SNAP) amplitude was 9.99 ± 5.48 μV; the mean conduction velocity was 49.01 ± 5.31 m/s. Significant correlations were found between age and height with DSN SNAP amplitude. Fifteen ATTRv pre-symptomatic carriers were examined. Sural nerve NCS were normal in 12/15 and revealed low/borderline values in three subjects. Considering our correction grid, we found an abnormal DNS amplitude in 9/15 subjects and low/borderline values in 2/15. In ATTRv, early detection of peripheral nerve damage is crucial to start a disease-modifying treatment. DSN may be easily and reliably included in the routine neurophysiological follow-up of ATTRv pre-symptomatic subjects.
本研究的目的是提供腓肠背侧神经（DSN）与年龄相关的正常参考值，并分析其在遗传性转甲状腺素蛋白淀粉样变性（ATTRv）症状前受试者随访中的应用。我们连续招募了ATTRv症状前携带者，对其进行了临床检查、心脏评估以及腓肠神经和DSN的神经传导研究。为了提供DSN的正常参考数据，将转诊至我们科室的健康对照者的神经生理学参数纳入线性回归分析，以检查年龄和身高的相对影响。然后得出一个校正表。我们收集了231名健康受试者：DSN感觉神经动作电位（SNAP）平均波幅为9.99±5.48μV；平均传导速度为49.01±5.31m/s。发现年龄和身高与DSN的SNAP波幅之间存在显著相关性。对15名ATTRv症状前携带者进行了检查。15名受试者中有12名的腓肠神经神经传导检查（NCS）结果正常，3名受试者的结果显示为低/临界值。根据我们的校正表，我们发现15名受试者中有9名的DNS波幅异常，2名受试者的结果为低/临界值。在ATTRv中，早期发现周围神经损伤对于启动疾病修饰治疗至关重要。DSN可以轻松且可靠地纳入ATTRv症状前受试者的常规神经生理学随访中。
